P1395: POSTERIOR REVERSIBLE ENCEPHALOPATHY SYNDROME AFTER PEDIATRIC HEMATOPOIETIC STEM CELL TRANSPLANTATION: SINGLE-CENTER EXPERIENCE
Authors: S. Zhumatayev, K. Yalcin, S. Celen, N. Azizova Gurbanzade, M. Mazen Jazazi, G. Karasu, V. Uygun, M. A. Yesilipek
Journal: HemaSphere (2022)

Background: Posterior reversible encephalopathy syndrome (PRES) is a reversible clinical-neuroradiological complication seen after allogeneic hematopoietic stem cell transplantation (allo-HSCT). Aims: This study aims to predict the risk factors that cause PRES in pediatric patients undergoing allo-HSCT and to evaluate the management of PRES. Methods: Forty-four patients were diagnosed with PRES out of 641 patients who underwent hematopoietic stem cell transplantation in Göztepe Medical Park Hospital Pediatric Bone Marrow Transplantation Unit, between April 2014 and November 2020, were evaluated retrospectively. Results: Characteristics of transplantation were presented in Table-1. The median PRES day of the patients was found to be 40 (1-363 days), and PRES occurred in the first 100 days in 84.4% of the cases.Thirty-three patients (75%) were using steroids at the time of PRES diagnosis, and concomitant hypertension was detected in 29 of these patients. Forty-three patients were using calcineurin inhibitors (CNI) as immunosuppressive therapy at the time of PRES. The median plasma cyclosporine (CSA) level measured closest to the time of PRES was detected 191 (65-650) ng/ml, and the median tacrolimus level was 15 (2.6-30) ng/ml. Antiepileptic, antihypertensive, and supportive therapy were initiated just after PRES. Thirteen patients (28.8%) were followed up in the intensive care unit. CNI was discontinued in 40 patients and instead of CNI, sirolimus was started in 8 patients, mycophenolate mofetil (MMF) in 22 patients, and ruxolitinib in 4 patients. In 2 of the 3 patients whose CNI was not discontinued, another type of CNI was used and the seizure did not recur. In one patient, the preparation was interrupted because the blood level was excessively high, and when the blood level was restored, the same preparation was continued, and seizures did not recur. Twenty-three patients (52.2%) had graft versus host disease (GvHD) at the time of PRES. Twelve patients (27.2%) who had no sign of GvHD at the time of PRES developed GvHD in the follow-up after the treatment alteration, and 5 of these patients died due to severe GvHD during the follow-up. When CNI treatments were discontinued in 7 patients with low-grade GvHD at the time of PRES, their GvHD stages increased and 4 patients died due to severe GVHD. Three patients who were followed up with severe GvHD during PRES died due to complications of GvHD. Image:Summary/Conclusion: As a result of this study, it is seen that the concomitant use of steroids with calcineurin inhibitors may increase the possibility of PRES. Therefore, the possibility of PRES development should be considered in cases where steroid addition is considered for the treatment of GvHD. The median level of calcineurin inhibitors measured during PRES is within the upper limits of normal and PRES findings can be seen even at low grades, suggesting that PRES may develop independently of the drug level. In addition, it has been observed that discontinuation of CNI in patients who develop PRES may lead to deterioration in GvHD findings and increase mortality. Therefore, in patients with high-grade GvHD, reintroduction of calcineurin inhibitors may be considered after PRES has been stabilized.